Referenced in: none

Automatically associated channels: Kir6.2

Title: Computed Tomography (CT) Scanning Facilitates Early Identification of Neonatal Cystic Fibrosis Piglets.

Authors: Antoine Guillon, Claire Chevaleyre, Celine Barc, Mustapha Berri, Hans Adriaensen, François Lecompte, Thierry Villemagne, Jérémy Pezant, Rémi Delaunay, Joseph Moënne-Loccoz, Patricia Berthon, Andrea Bähr, Eckhard Wolf, Nikolai Klymiuk, Sylvie Attucci, Reuben Ramphal, Pierre Sarradin, Dominique Buzoni-Gatel, Mustapha Si-Tahar, Ignacio Caballero

Journal, date & volume: PLoS ONE, 2015 , 10, e0143459

PubMed link: http://www.ncbi.nlm.nih.gov/pubmed/26600426

Abstract
Cystic Fibrosis (CF) is the most prevalent autosomal recessive disease in the Caucasian population. A cystic fibrosis transmembrane conductance regulator knockout (CFTR-/-) pig that displays most of the features of the human CF disease has been recently developed. However, CFTR-/- pigs presents a 100% prevalence of meconium ileus that leads to death in the first hours after birth, requiring a rapid diagnosis and surgical intervention to relieve intestinal obstruction. Identification of CFTR-/- piglets is usually performed by PCR genotyping, a procedure that lasts between 4 to 6 h. Here, we aimed to develop a procedure for rapid identification of CFTR-/- piglets that will allow placing them under intensive care soon after birth and immediately proceeding with the surgical correction.Male and female CFTR+/- pigs were crossed and the progeny was examined by computed tomography (CT) scan to detect the presence of meconium ileus and facilitate a rapid post-natal surgical intervention. Genotype was confirmed by PCR. CT scan presented a 94.4% sensitivity to diagnose CFTR-/- piglets. Diagnosis by CT scan reduced the birth-to-surgery time from a minimum of 10 h down to a minimum of 2.5 h and increased the survival of CFTR-/- piglets to a maximum of 13 days post-surgery as opposed to just 66 h after later surgery.CT scan imaging of meconium ileus is an accurate method for rapid identification of CFTR-/- piglets. Early CT detection of meconium ileus may help to extend the lifespan of CFTR-/- piglets and, thus, improve experimental research on CF, still an incurable disease.